People with
Darier’s disease can go into depression
It is a rare disorder, inherited in an autosomal
dominant pattern
New Delhi, 04
December 2017: Recently, a case of Darier’s disease was reported from a
city in North India. So far, about 15 cases have occurred in India, with this
being the first in Uttar Pradesh. The disease is so rare that it affects only
one out of one lakh people. Although the disease does not kill, it can be
potentially debilitating for the person in question and put them on lifelong
precautionary treatment.
Darier
disease is a skin condition characterized by wart-like blemishes on the body.
The blemishes are usually yellowish in color, hard to the touch, mildly greasy,
and can emit a strong odor. The most common sites for blemishes are the scalp,
forehead, upper arms, chest, back, knees, elbows, and behind the ear. The
mucous membranes can also be affected, with blemishes on the roof of the mouth
(palate), tongue, inside of the cheek, gums, and throat.
Speaking
about this, Padma Shri Awardee Dr K K Aggarwal, National President Indian
Medical Association (IMA) and President Heart Care Foundation of India (HCFI)
and Dr RN Tandon – Honorary Secretary General IMA in a joint statement, said,
"The wart-like blemishes in this disease usually appear in late childhood
to early adulthood. Without precautionary measures, it can become more severe
over time with the affected people experiencing flare-ups alternating with
periods when they have fewer blemishes. The appearance of the blemishes is
influenced by environmental factors such as heat and humidity; UV light; minor
injury or friction; and ingestion of certain medications. At times, people with
Darier disease may have neurological disorders; and issues with learning and
behavior as well. This condition is inherited in an autosomal dominant pattern,
which means one copy of the altered gene in each cell is sufficient to cause
the disorder.”
Itching is
very common in those with this condition. The affected skin may smell
unpleasant, particularly in moist areas. This is probably caused by increased
numbers of ordinary skin bacteria growing in the affected skin.
Adding
further, Dr Aggarwal, said, “The diagnosis of Darier’s disease can often
be made on the appearance of the rash and the fact that it runs in
families. To confirm it, a small sample of skin (a biopsy) can be removed
under a local anesthetic and examined under the microscope in the laboratory.
Genetic testing to identify a mutation in the ATP2A2 gene can be used to confirm
the diagnosis as well.”
Some
precautions to be taken for those with this disease include the following.
- Avoid stress, excessive
humidity, heat and tight clothes and use moisturizers, sun block and wear
the right type of clothes which absorb sweat and keep the body cool.
- For localized lesions,
dermabrasion can be done. Topical retinoids are also prescribed and are
beneficial.
- If the patient has
secondary bacterial infection, then antibiotics are prescribed. Antiviral
medications are prescribed if the patient has herpes simplex.
- For severe symptoms, oral
retinoids can be prescribed, but these medications have serious side
effects and need to be used with caution. Topical or oral antibiotics can
be prescribed during flare ups.
- Using sunscreen and
taking vitamin C tablets helps in preventing flare ups in some patients.